Cheshire Helen, Stather Philip, Vorster Johan
Department of Dermatology, Kettering General Hospital, Kettering, United Kingdom.
J Dermatol Case Rep. 2009 Nov 28;3(3):41-3. doi: 10.3315/jdcr.2009.1032.
Acrodermatitis enteropathica is characterised by inflamed patches of dry red skin which then become crusted and blistered before revealing a pustulent eroded lesion. Typically these patches start near the body's orifices before migrating to other sites, however in this patient the presentation was atypical thus delaying the diagnosis.
We report a case of an atypical presentation of acrodermatitis enteropathica (AE) due to acquired zinc deficiency in a 65 year old female patient with a previous diagnosis of histologically confirmed Darier's disease. This patient's rash began on the limbs and trunk before progressing to include the perineum, oral mucosa and nose. Diffuse loss of hair on scalp, eyebrows and eyelashes was observed. Acrodermatitis enteropathica typically presents in infants, either due to an autosomal recessive genetic disorder, or after the cessation of breast feeding. In adults acquired zinc deficiency can be caused by glucagonoma syndrome, poor nutritional state, intestinal malabsorption, nephrotic syndrome and after major trauma (i.e. burns or significant surgery). In our patient low zinc levels confirmed hypozincaemia and the diagnosis of acrodermatitis enteropathica. The patient started oral zinc replacement. Within a few days her symptoms began to improve, and her hair has started to grow.
We believe this to be an unusual presentation of acrodermatitis enteropathica due to a probable dietary zinc deficiency in a lady with pre-existing Darier's disease which may possibly have influenced the uncharacteristic clinical presentation.
肠病性肢端皮炎的特征是皮肤出现干燥、发红的炎症斑块，随后结痂、起泡，进而形成脓疱性糜烂性病变。通常这些斑块始于身体孔窍附近，之后蔓延至其他部位，然而该患者的表现不典型，从而延误了诊断。
我们报告一例65岁女性患者，因获得性锌缺乏导致肠病性肢端皮炎（AE）的非典型表现，该患者先前经组织学确诊为 Darier 病。该患者的皮疹始于四肢和躯干，之后发展至包括会阴、口腔黏膜和鼻部。观察到头皮、眉毛和睫毛弥漫性脱发。肠病性肢端皮炎通常见于婴儿，原因要么是常染色体隐性遗传病，要么是停止母乳喂养后。在成人中，获得性锌缺乏可由胰高血糖素瘤综合征、营养状况差、肠道吸收不良、肾病综合征以及重大创伤（如烧伤或大手术）引起。在我们的患者中，低锌水平证实了低锌血症及肠病性肢端皮炎的诊断。患者开始口服补锌治疗。数天内其症状开始改善，头发也开始生长。
我们认为这是一例因可能的膳食锌缺乏导致的肠病性肢端皮炎的不寻常表现，该患者患有 Darier 病，这可能影响了其非典型的临床表现。
